1.Clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis: a retrospective analysis.
Rui-Yan WANG ; Hui CHEN ; Zhi-Xin HUANG ; Yong CHEN ; Jian-Min ZHONG
Chinese Journal of Contemporary Pediatrics 2023;25(10):1034-1039
OBJECTIVES:
To investigate the clinical effect of different immunosuppressive treatment regimens in children with ocular myasthenia gravis (OMG).
METHODS:
A retrospective analysis was conducted on 130 children with OMG who were treated in the Department of Neurology, Jiangxi Children's Hospital, from February 2018 to February 2023. According to the treatment regimen, they were divided into four groups: glucocorticoid (GC) group (n=29), mycophenolate mofetil (MMF) group (GC+MMF; n=33), methotrexate (MTX) group (GC+MTX; n=30), and tacrolimus (FK506) group (GC+FK506; n=38). Treatment outcomes and adverse reactions were compared among the groups.
RESULTS:
After 3 months of treatment, the FK506 group had significantly lower scores of Myasthenia Gravis Quantitative Scale and Myasthenia Gravis-Specific Activities of Daily Living than the other three groups (P<0.05). After 3 months of treatment, the FK506 group had a significantly lower dose of prednisone than the GC group, and after 6 and 9 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower dose of prednisone than the GC group (P<0.05). After 12 months of treatment, the MMF, MTX, and FK506 groups had a significantly lower incidence rate of GC-related adverse reactions than the GC group (P<0.05).
CONCLUSIONS
For children with OMG, the addition of various immunosuppressants can reduce the dosage of GC and adverse reactions. Among them, FK506 shows superior efficacy compared to other immunosuppressants in the early treatment of OMG.
Humans
;
Child
;
Prednisone/adverse effects*
;
Tacrolimus/adverse effects*
;
Retrospective Studies
;
Activities of Daily Living
;
Immunosuppressive Agents/adverse effects*
;
Myasthenia Gravis/drug therapy*
;
Glucocorticoids/therapeutic use*
;
Mycophenolic Acid/adverse effects*
2.Expression of IGLL1 Gene and Its Clinical Significance in Pediatric T-ALL.
Shui-Yan WU ; Xin-Ran CHU ; Qi JI ; Xiao-Chen LIN ; Zhen-Jiang BAI ; Jian-Qin LI ; Jian PAN ; Zi-Xing CHEN ; Shao-Yan HU
Journal of Experimental Hematology 2023;31(4):999-1004
OBJECTIVE:
To detect the relative expression of IGLL1 (immunoglobulin lambda-like polypeptide 1) mRNA in bone marrow of children with T-cell acute lymphoblastic leukemia (T-ALL), and analyze its correlation with the clinical characteristics and prognosis of the patients, so as to clarify the clinical significance of IGLL1 in pediatric T-ALL patients.
METHODS:
A total of 56 pediatric T-ALL patients hospitalized in Children's Hospital of Soochow University from June 2012 to December 2017 and treated with CCLG-ALL 2008 regimen were selected. Transcriptome sequencing technology was used to detect the transcription level of IGLL1 gene in children with T-ALL. According to 25% of the IGLL1 transcription level (cutoff value:448), the enrolled children were divided into IGLL1 low expression group (17 cases) and IGLL1 high expression group (39 cases). Combined with clinical data, the correlation between the expression level of IGLL1 and prognosis of the patients was analyzed.
RESULTS:
The comparative analysis showed that the transcription level of IGLL1 was not correlated with the clinical characteristics of the patients, such as sex, age, bone marrow blast, white blood cell (WBC) count at initial diagnosis. The 5-year OS rate of patients with high IGLL1 expression was significantly higher than that of patients with low IGLL1 expression (76.9%±6.7% vs 47.1%±12.1%, P =0.018). Further comparison of relapse-free survival (RFS) rate between the two groups showed that the 5-year RFS rate of patients with high IGLL1 expression was higher than that of patients with low IGLL1 expression, but the difference between the two groups was not statistically significant (P =0.095). Multivariate COX analysis was conducted on common clinical prognostic factors (age, sex, WBC count at diagnosis, prednisone response on the 7th day, bone marrow response on the 15th day after treatment) and IGLL1 expression level, and the results showed that IGLL1 expression (P =0.012) and prednisone response (P =0.017) were independent risk factors for overall survival in pediatric T-ALL patients.
CONCLUSION
In pediatric T-ALL, the OS rate of children with high expression of IGLL1 gene was significantly higher than that of children with low expression of IGLL1 gene, and the expression level of IGLL1 gene was an independent factor affecting the survival of children with T-ALL, which suggests that IGLL1 is a marker of good clinical prognosis of children with T-ALL.
Child
;
Humans
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Clinical Relevance
;
Disease-Free Survival
;
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/genetics*
;
Prednisone/therapeutic use*
;
Prognosis
;
Recurrence
;
Immunoglobulin Light Chains, Surrogate/genetics*
3.Composite B-cell and T-cell lymphomas: clinical, pathological, and molecular features of three cases and literature review.
Xueli JIN ; Hui LIU ; Jing LI ; Xibin XIAO ; Xianggui YUAN ; Panpan CHEN ; Boxiao CHEN ; Yun LIANG ; Fengbo HUANG
Journal of Zhejiang University. Science. B 2023;24(8):711-722
Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.
Humans
;
Rituximab/therapeutic use*
;
Vincristine/therapeutic use*
;
Prednisone/therapeutic use*
;
Epstein-Barr Virus Infections/drug therapy*
;
Herpesvirus 4, Human
;
Neoplasm Recurrence, Local
;
Lymphoma, T-Cell/drug therapy*
;
Cyclophosphamide/therapeutic use*
;
Lymphoma, Large B-Cell, Diffuse/pathology*
;
Doxorubicin/therapeutic use*
;
Lymphadenopathy/drug therapy*
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
4.Treatment conundrum: A case of recalcitrant Epidermolysis Bullosa Acquisita (EBA) in a 50-year-old Filipino male
Danelle Anne L. Santos, MD ; Aira Monica R. Abella, MD ; Danica-Grace Tungol, MD, DPDS ; Leilani R. Senador, MD, FPDS
Journal of the Philippine Dermatological Society 2023;32(1):31-34
Introduction:
Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune blistering disease which presents in the skin and mucous
membranes. The decrease in anchoring fibrils in the basement membrane zone causes separation of the epidermis from the dermis,
resulting in its blistering presentation. The treatment plan will depend on the severity of the disease. The first-line treatment for mild
EBA includes topical corticosteroids and immunomodulators such as dapsone and colchicine; while severe cases of EBA may be given
intravenous immunoglobulins, systemic steroids, and immunosuppressants such as azathioprine and cyclophosphamide.
Case Report:
This is a case of a 50-year-old Filipino male who presented with a 2-year history of vesicles and tense bullae which evolved
into papules, plaques and erosions with scarring and milia formation on the scalp and trauma-prone areas of the trunk and extremities.
Clinical examination revealed multiple, well-defined, irregularly shaped erythematous papules and plaques with crusts, scales, erosions, pearl-like milia and scarring on the chest, back, upper, and lower extremities. The oral mucosa was moist with some ulcers on the
tongue. Histopathologic examination using Hematoxylin and Eosin (H&E) stain revealed the absence of the epidermis with retention of
dermal papillae suggestive of subepidermal clefting. Further examination with direct immunofluorescence (DIF) revealed monoclonal
immunoglobulin (IgG) deposits demonstrating an intense linear fluorescent band at the dermoepidermal junction, consistent with Epidermolysis Bullosa Acquisita. Overall, the combined administration of prednisone, azathioprine, and colchicine resulted only in transient
and incomplete resolution of lesions in this case of EBA.
Conclusion
The management of EBA is mostly supportive with the goal of minimizing complications. Combination treatments using
steroids, colchicine, and azathioprine have been reported with various results. Its management remains challenging as most cases are
refractory to treatment.
Epidermolysis Bullosa Acquisita
;
bullous disease
;
azathioprine
;
colchicine
;
prednisone
5.Clinicopathologic characteristics and prognostic analysis of testicular diffuse large B-cell lymphoma.
Yue WANG ; Zi Yang SHI ; Qing SHI ; Shuo WANG ; Mu Chen ZHANG ; Rong SHEN ; Yang HE ; Hui Ling QIU ; Hong Mei YI ; Lei DONG ; Li WANG ; Shu CHENG ; Peng Peng XU ; Wei Li ZHAO
Chinese Journal of Hematology 2023;44(4):321-327
Objective: To analyze the clinicopathologic characteristics and prognosis of testicular diffuse large B-cell lymphoma (DLBCL) . Methods: A retrospective analysis was performed on 68 patients with testicular DLBCL admitted to Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine from October 2001 to April 2020. The gene mutation profile was evaluated by targeted sequencing (55 lymphoma-related genes) , and prognostic factors were analyzed. Results: A total of 68 patients were included, of whom 45 (66.2% ) had primary testicular DLBCL and 23 (33.8% ) had secondary testicular DLBCL. The proportion of secondary testicular DLBCL patients with Ann Arbor stage Ⅲ-Ⅳ (P<0.001) , elevated LDH (P<0.001) , ECOG score ≥ 2 points (P=0.005) , and IPI score 3-5 points (P<0.001) is higher than that of primary testicular DLBCL patients. Sixty-two (91% ) patients received rituximab in combination with cyclophosphamide, adriamycin, vincristine, and prednisone (R-CHOP) -based first-line regimen, whereas 54 cases (79% ) underwent orchiectomy prior to chemotherapy. Patients with secondary testicular DLBCL had a lower estimated 5-year progression-free survival (PFS) rate (16.5% vs 68.1% , P<0.001) and 5-year overall survival (OS) rate (63.4% vs 74.9% , P=0.008) than those with primary testicular DLBCL, and their complete remission rate (57% vs 91% , P=0.003) was also lower than that of primary testicular DLBCL. The ECOG scores of ≥2 (PFS: P=0.018; OS: P<0.001) , Ann Arbor stages Ⅲ-Ⅳ (PFS: P<0.001; OS: P=0.018) , increased LDH levels (PFS: P=0.015; OS: P=0.006) , and multiple extra-nodal involvements (PFS: P<0.001; OS: P=0.013) were poor prognostic factors in testicular DLBCL. Targeted sequencing data in 20 patients with testicular DLBCL showed that the mutation frequencies of ≥20% were PIM1 (12 cases, 60% ) , MYD88 (11 cases, 55% ) , CD79B (9 cases, 45% ) , CREBBP (5 cases, 25% ) , KMT2D (5 cases, 25% ) , ATM (4 cases, 20% ) , and BTG2 (4 cases, 20% ) . The frequency of mutations in KMT2D in patients with secondary testicular DLBCL was higher than that in patients with primary testicular DLBCL (66.7% vs 7.1% , P=0.014) and was associated with a lower 5-year PFS rate in patients with testicular DLBCL (P=0.019) . Conclusion: Patients with secondary testicular DLBCL had worse PFS and OS than those with primary testicular DLBCL. The ECOG scores of ≥2, Ann Arbor stages Ⅲ-Ⅳ, increased LDH levels, and multiple extra-nodal involvements were poor prognostic factors in testicular DLBCL. PIM1, MYD88, CD79B, CREBBP, KMT2D, ATM, and BTG2 were commonly mutated genes in testicular DLBCL, and the prognosis of patients with KMT2D mutations was poor.
Male
;
Adult
;
Humans
;
Prognosis
;
Retrospective Studies
;
Myeloid Differentiation Factor 88
;
China/epidemiology*
;
Testicular Neoplasms/drug therapy*
;
Cyclophosphamide
;
Rituximab/therapeutic use*
;
Lymphoma, Large B-Cell, Diffuse/drug therapy*
;
Prednisone/therapeutic use*
;
Doxorubicin/therapeutic use*
;
Vincristine/therapeutic use*
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Immediate-Early Proteins/therapeutic use*
;
Tumor Suppressor Proteins
6.Dose-adjusted EPOCH-R vs. R-CHOP in frontline management of Waldeyer's ring diffuse large B-cell lymphoma: a retrospective study from a single institution.
Yuanzheng LIANG ; Xindi LIU ; Jing YANG ; Henan WANG ; Yingshi PIAO ; Liqiang WEI ; Liang WANG
Chinese Medical Journal 2023;136(2):167-175
BACKGROUND:
To compare the efficacy and safety of dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin plus rituximab (DA-EPOCH-R) with standard rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) in Waldeyer's ring diffuse large B-cell lymphoma (WR-DLBCL) at a single institution.
METHODS:
This retrospective study included 115 newly diagnosed patients with WR-DLBCL, of whom 68 patients received R-CHOP, and 47 patients received DA-EPOCH-R as their first-line treatment. The baseline features of the two groups were well balanced using a 1:1 propensity score matching method, and a total of 84 cases were obtained, including respective 42 cases in the R-CHOP and DA-EPOCH-R groups, for further survival and prognosis analysis. The primary objectives included progression-free survival (PFS) and overall survival (OS).
RESULTS:
During a median follow-up of 45 months, there were nine (21.4%) deaths in the R-CHOP group and two (4.8%) in the DA-EPOCH-R group. Kaplan-Meier analysis showed statistically significant improvements in PFS and OS in patients with DA-EPOCH-R compared with those treated with R-CHOP (log-rank test, P = 0.025 and P = 0.035, respectively). The 2-year PFS and OS rates in the DA-EPOCH-R group were 90.1% (95% confidence interval [CI]: 81.4-99.8%) and 95.2% (95% CI: 89.0-100.0%), respectively, and 80.5% (95% CI: 69.3-93.6%) and 90.5% (95% CI: 52.8-99.8%) in the R-CHOP group. Patients without B symptoms and elevated lactate dehydrogenase levels had a higher PFS in the DA-EPOCH-R group, with P values of 0.038 (hazard ratio [HR]: 0.11; 95% CI: 0.01-0.88) and 0.042 (HR: 0.19; 95% CI: 0.04-0.94), respectively. There were no statistically significant differences in clinical responses and treatment-related toxicities between the two groups.
CONCLUSION
Compared with patients received R-CHOP, those treated by DA-EPOCH-R had superior PFS, OS, and controlled toxicity in patients with WR-DLBCL.
Humans
;
Rituximab/therapeutic use*
;
Vincristine/therapeutic use*
;
Retrospective Studies
;
Prednisone/therapeutic use*
;
Etoposide/therapeutic use*
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Lymphoma, Large B-Cell, Diffuse/drug therapy*
;
Cyclophosphamide/therapeutic use*
;
Doxorubicin/therapeutic use*
7.A case of duodenal ulcer as prominent manifestation of IgG4-related disease.
Min FENG ; Zhe CHEN ; Yong Jing CHENG
Journal of Peking University(Health Sciences) 2023;55(6):1125-1129
A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.
Aged
;
Humans
;
Male
;
Abdominal Pain/drug therapy*
;
Acute Disease
;
Bilirubin
;
Duodenal Ulcer/etiology*
;
Immunoglobulin G
;
Immunoglobulin G4-Related Disease/diagnosis*
;
Pancreatitis/drug therapy*
;
Positron Emission Tomography Computed Tomography
;
Prednisone/therapeutic use*
;
Pruritus/drug therapy*
8.Clinical characteristics and prognostic features of 63 HIV-associated diffuse large B-cell lymphoma: a single-center real-world study in China.
Chao Yu WANG ; Jun LIU ; Xi Ping LIANG ; Bing Lin GUO ; Ren Zhi HU ; Yao LIU
Chinese Journal of Hematology 2022;43(3):203-208
Objective: This study aimed to look into the clinical characteristics and prognosis of patients with human immunodeficiency virus (HIV) -associated diffuse large B-cell lymphoma (DLBCL) . Methods: Retrospective review of the clinical data of 63 HIV-infected patients with DLBCL diagnosed at Chongqing University Cancer Hospital between July 2008 and August 2021. The Kaplan-Meier method was used to calculate survival curves, and the log-rank test method was used to compare survival between groups. The Cox proportional hazards model was used for multivariate analysis. Results: In 63 patients with HIV-associated DLBCL, 57 (90.5% ) were men, and the median age was 49 (23-87) years. The most common pathological subtype was the germinal center B-cell-like lymphoma (74.6% ) ; 46.0% (29/63) were combined with extranodal lesions. Seventeen of 63 (27.0% ) patients had large masses (≥7.5 cm) . Twenty of 63 (31.7% ) patients had B symptoms. The median CD4(+) T cell count was 203 (4-1022) ×10(6)/L. A total of 49% (25/51) patients had CD4(+) cell count <200×10(6)/L, 56.9% (33/58) had high (3-5) International Prognostic Index (IPI) scores, and 43.1% (25/58) had low (0-2) IPI scores. Further, 78% (46/59) were diagnosed with Ann Arbor Stage Ⅲ/Ⅳ, and 25.4% (16/63) didn't receive chemotherapy. A total of 22.2% (14/63) of patients received less than four cycles of chemotherapy, and 52.4% (33/63) received four or more cycles of chemotherapy. Among patients undergoing chemotherapy, 61.7% (29/47) received R-CHOP-like regimens, and 38.3% (18/47) used CHOP-like regimens. The 1-, 2-, 3-, and 5-year overall survival (OS) rates were 65.0% , 53.8% , 47.1% , and 43.5% , respectively. Univariate analysis revealed that age ≥ 60 years (P=0.012) , Eastern Cooperative Oncology Gruop Performance Status (ECOG-PS) score 2-4 points (P=0.043) , IPI score 3-5 points (P=0.001) , β(2)-MG elevation (≥5.5 mg/L) (P=0.007) , and systemic chemotherapy cycles less than four times (P<0.001) were the negative prognostic factors affecting the OS of patients. The Cox multivariate analysis depicted that age ≥60 years (HR=2.272, 95% CI 1.110-4.651, P=0.025) , IPI score 3-5 points (HR=3.562, 95% CI 1.794-7.074, P<0.001) , ECOG-PS score 2-4 points (HR=2.675, 95% CI 1.162-6.153, P=0.021) , and number of cycles of chemotherapy<4 (HR=0.290, 95% CI 0.176-0.479, P<0.001) were independent risk factors for adverse prognosis of OS. Conclusion: HIV-associated DLBCL is the most common HIV-related tumor, is most commonly seen in men, and has a high 1-year mortality rate. Chemotherapy combined with antiretroviral therapy can improve patient prognosis.
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Cyclophosphamide/therapeutic use*
;
Doxorubicin/therapeutic use*
;
HIV Infections
;
Humans
;
Lymphoma, Large B-Cell, Diffuse/drug therapy*
;
Male
;
Middle Aged
;
Prednisone/therapeutic use*
;
Prognosis
;
Retrospective Studies
;
Rituximab/therapeutic use*
;
Survival Rate
;
Vincristine/therapeutic use*
9.Predictors of efficacy of corticosteroid switching from abiraterone plus prednisone to dexamethasone in patients with metastatic castration-resistant prostate cancer.
Yu-Chao NI ; Jin-Ge ZHAO ; Meng-Ni ZHANG ; Yi-Jun ZHANG ; Zhen-Yu YANG ; Ni CHEN ; Jun-Ru CHEN ; Peng-Fei SHEN ; Guang-Xi SUN ; Xing-Ming ZHANG ; Yong-Hong LI ; Hao ZENG
Asian Journal of Andrology 2022;24(2):154-160
Corticosteroid switching can reverse abiraterone resistance in some patients with metastatic castration-resistant prostate cancer (mCRPC). Here, we investigated the potential biomarkers for predicting the efficacy of corticosteroid switching during treatment with abiraterone acetate (AA). We retrospectively analyzed 101 mCRPC patients receiving corticosteroid switching from West China Hospital and Sun Yat-Sen University Cancer Center between January 2016 and December 2018. All cases received AA plus prednisone as first-line therapy during mCRPC. Primary end points were biochemical progression-free survival (bPFS) and overall survival (OS). The risk groups were defined based on multivariate analysis. A total of 42 (41.6%) and 25 (24.8%) patients achieved 30% and 50% decline in prostate-specific antigen (PSA), respectively, after corticosteroid switching. The median bPFS and median OS on AA plus dexamethasone were 4.9 (95% confidence interval [CI]: 3.7-6.0) months and 18.8 (95% CI: 16.2-30.2) months, respectively. Aldo-keto reductase family 1 member C3 (AKR1C3) expression (hazard ratio [HR]: 2.15, 95% Cl: 1.22-3.80, P = 0.008) and baseline serum alkaline phosphatase (ALP; HR: 4.95, 95% Cl: 2.40-10.19, P < 0.001) were independent predictors of efficacy before corticosteroid switching in the multivariate analysis of bPFS. Only baseline serum ALP >160 IU l-1 (HR: 3.41, 95% Cl: 1.57-7.38, P = 0.002) together with PSA level at switch ≥50 ng ml-1 (HR: 2.59, 95% Cl: 1.22-5.47, P = 0.013) independently predicted poorer OS. Based on the predictive factors in multivariate analysis, we developed two risk stratification tools to select candidates for corticosteroid switching. Detection of serum ALP level, PSA level, and tissue AKR1C3 expression in mCRPC patients could help make clinical decisions for corticosteroid switching.
Abiraterone Acetate/therapeutic use*
;
Adrenal Cortex Hormones/therapeutic use*
;
Androstenes
;
Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
;
Dexamethasone/therapeutic use*
;
Disease-Free Survival
;
Humans
;
Male
;
Prednisone/therapeutic use*
;
Prostate-Specific Antigen
;
Prostatic Neoplasms, Castration-Resistant/pathology*
;
Retrospective Studies
;
Treatment Outcome
10.Clinical assessment of moderate-dose glucocorticoid in the treatment of recurrence of primary nephrotic syndrome in children: a prospective randomized controlled trial.
Juan TU ; Chao-Ying CHEN ; Hai-Yun GENG ; Hua-Rong LI ; Hua XIA ; Yuan LIN ; Tian-Tian LIN ; Jin-Shan SUN
Chinese Journal of Contemporary Pediatrics 2022;24(5):466-471
OBJECTIVES:
To study the clinical effect and adverse drug reactions of different doses of glucocorticoid (GC) in the treatment of children with recurrence of steroid-sensitive nephrotic syndrome (SSNS).
METHODS:
A total of 67 children who were hospitalized and diagnosed with SSNS recurrence in the Department of Nephrology, Children's Hospital, Capital Institute of Pediatrics, from November 2017 to December 2019 were enrolled. They were randomly divided into a moderate-dose GC group (32 children) and a full-dose GC group (35 children). The two groups were compared in terms of urinary protein clearance, recurrence rate within 6 months, and incidence rate of GC-associated adverse reactions.
RESULTS:
There was no significant difference in the urinary protein clearance rate between the moderate-dose GC and full-dose GC groups (91% vs 94%, P>0.05). There was also no significant difference in the recurrence rate within 6 months between the two groups (41% vs 36%, P>0.05). At 6 months of follow-up, compared with the full-dose GC group, the moderate-dose GC group had a significantly lower cumulative dose of prednisone [(87±18) mg/kg vs (98±16) mg/kg, P=0.039] and a significantly lower proportion of children with an abnormal increase in body weight (6% vs 33%, P=0.045). The logistic regression analysis showed that prednisone dose ≥10 mg/alternate day at enrollment was a risk factor for recurrence within 6 months in children with SSNS (P=0.018).
CONCLUSIONS
For children with SSNS recurrence, moderate-dose GC has similar effects to full-dose GC in the remission induction rate and the recurrence rate within 6 months, with a lower cumulative dose and fewer GC-associated adverse reactions within 6 months than full-dose GC.
Child
;
Glucocorticoids/therapeutic use*
;
Humans
;
Nephrotic Syndrome/drug therapy*
;
Prednisone/adverse effects*
;
Prospective Studies
;
Remission Induction


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